POEMS

What is POEMS syndrome?

POEMS syndrome is a rare type of plasma cell disorder that can affect multiple systems in the body. It’s named after the five common features of the syndrome:

• Polyneuropathy – nerve damage to various peripheral nerves (the nerves outside the brain and spinal cord) which control muscles and sensation. Also known as peripheral neuropathy
• Organomegaly – enlargement of organs, such as the liver, spleen or lymph nodes
• Endocrinopathy – abnormal function of endocrine (hormone-producing) glands
• Monoclonal plasma cell disorder – such as myeloma or another plasma cell disorder
• Skin changes – certain skin changes are characteristic in POEMS syndrome, such as hyperpigmentation (darkening of an area of the skin or nails)

How is POEMS syndrome diagnosed?

To diagnose POEMS syndrome, the doctor will need to perform a thorough physical examination where they will look for: skin and hair changes; evidence of fluid build-up; enlargement of specific glands and organs such as lymph glands, liver and/or the spleen; signs of optic nerve swelling; signs of nerve damage. Blood and urine tests, imaging scans and biopsies may also be carried out to confirm the diagnosis.

How is POEMS syndrome treated?

Treatment of POEMS syndrome will depend on several factors including the type of underlying plasma cell disorder (e.g. MGUS or myeloma), the main symptoms present and organs affected and the age and general fitness of the patient. Treatment may include chemotherapy and/or radiotherapy.

Further reading